Extracorporeal Photopheresis for the Treatment of Cutaneous T-Cell Lymphoma

Abstract

C utaneous T-cell lymphoma (CTCL) is the general term used to describe extracutaneous non-Hodgkin's lymphomas characterized by monoclonal expansion of skin-homing malignant T cells that can further be defined by the use of immunophenotypic markers and the presence of specific rearrangements of the T-cell receptor.' The most frequently diagnosed form of CTCL is mycosis fungoides (MF) and its leukemic variant, Sezary syndrome (SS).2 MF most often presents as chronic eczematous or psoriasiform patches or plaques and can remain stable for many years. In some patients, MF progresses to involve lymph nodes, peripheral blood, bone marrow, and visceral organs.' SS may present de novo with persistent exfoliative erythroderma accompanied by staphylococcal colonization, perivascular atypical lymphocytes in the dermis, and ;:::1000 atypical T lymphocytes/mm' in the blood, a criterion recently defined as a B2 blood rating by the International Society for Cutaneous Lymphomas.Y Extracorporeal photopheresis (ECP) combines blood apheresis with ultraviolet-A irradiation of psoralen-photosensitized peripheral blood mononuclear cells. ECP was first introduced in 1987 for the treatment of erythrodermic MF and SS. ECP was the first therapy to receive FDA approval for the treatment of CTCL based on a multicenter trial. 6 Twenty-seven of 37 patients (76%) achieved a 25%-100% reduction in their erythroderma skin score. The Yale-New Haven Hospital experience has been reviewed by Heald et at.7 who treated 32 patients with ECP. Of 19 erythrodermic patients who were treated with ECP as their first therapy at Yale, five had greater than 75% improvement of their skin score, ten had 25%-50% improvement, and four had less than 25% improvement. The majority of patients had improved quality of life. Heald et al. 8 also evaluated long-term followup in 29 original CTCL patients in the original multicenter study. Their mean survival from the time of diagnosis was 60.3 months and from beginning of therapy it was 47.9 months, which exceeds their expected mean overall survival of 24-30 months reported in the litera

Extracorporeal Photopheresis in the Treatment of Mycosis Fungoides and Sézary Syndrome

“…In one report, ECP monotherapy showed a 40% response rate in patients with stage III/IV MF/SS in contrast to a 57% response rate in those treated with a combination of ECP plus IFN alfa, bexarotene, or GM-CSF. 62 In a retrospective review of 98 patients with SS treated with ECP (>3 months) and 1 or more systemic immunostimulatory agents (IFN gamma, IFN alfa, GM-CSF, systemic retinoids), Raphael and colleagues 63 at the University of Pennsylvania reported a significant improvement in 73 patients (75%) with 29 CRs (30%).…”

“…Since FDA approval of ECP in 1988, there has been minor variability in the 2-day cycle every 4 weeks in the treatment of MF/SS. Duvic and colleagues 42 found no increased response rate using an accelerated regimen of one 2-day cycle every 2 weeks to treat a small cohort of patients with MF/SS. More recently, Siakantaris and colleagues 43 from Greece published their retrospective experience (N 5 18) using an accelerated treatment schedule of one cycle of ECP every week for 1 month, followed by 1 cycle of ECP every 2 weeks for 2 months, and then one cycle of ECP every month.…”

Sézary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)

“…[148][149][150] Efficacy in treating certain clinical stages (IB, IIA, III, and IVA) and skin stages (patch/plaque [T 2 ] and erythroderma [T 4 ]) of MF and SS with ECP is favorable, although randomized trials comparing ECP with other standard therapies are needed. [148][149][150] The overall RR for ECP monotherapy has ranged from 36% in an intention-to-treat analysis 149 to 10%, 17%, 50%, 80%, and 83% in those patients with MF/SS who have been on ECP for at least 3 months. 84,90,149,151 Edelson et al 152 reported a response ( $ 25%) in 83% (24 of 29) of patients with E-MF (blood involvement not given).…”

“…[148][149][150] The overall RR for ECP monotherapy has ranged from 36% in an intention-to-treat analysis 149 to 10%, 17%, 50%, 80%, and 83% in those patients with MF/SS who have been on ECP for at least 3 months. 84,90,149,151 Edelson et al 152 reported a response ( $ 25%) in 83% (24 of 29) of patients with E-MF (blood involvement not given). Those studies in which SS has been clearly defined and an OR defined as at least 50% clearing are shown in Table I.…”

Central nervous system involvement in patients with mycosis fungoides and cutaneous large-cell transformation

“…13 LCT of MF is associated with a more aggressive clinical course, extracutaneous disease, and worse prognosis with decreased survival. 14, 15 We report 5 additional patients with MF, LCT, and CNS disease to better define their presentation, treatment, and prognosis.…”